Based on the CBC and electrophoresis showing HbS predominance with no HbA and a sickled peripheral smear, what is the most likely diagnosis?

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Multiple Choice

Based on the CBC and electrophoresis showing HbS predominance with no HbA and a sickled peripheral smear, what is the most likely diagnosis?

Having HbS as the major hemoglobin with no HbA on electrophoresis points to homozygous sickle cell disease, because both beta-globin genes carry the sickle mutation and little or no normal HbA is produced. The sickled cells seen on the smear support this, since HbS polymerizes and deforms red cells under low oxygen, causing chronic hemolysis and vaso-occlusion typical of SCD. If this were sickle cell trait, HbA would still be present because only one beta-globin gene carries HbS, so HbA and HbS coexist. Hemoglobin C disease would show HbC predominance rather than HbS, and iron deficiency anemia would not show HbS at all.

Having HbS as the major hemoglobin with no HbA on electrophoresis points to homozygous sickle cell disease, because both beta-globin genes carry the sickle mutation and little or no normal HbA is produced. The sickled cells seen on the smear support this, since HbS polymerizes and deforms red cells under low oxygen, causing chronic hemolysis and vaso-occlusion typical of SCD. If this were sickle cell trait, HbA would still be present because only one beta-globin gene carries HbS, so HbA and HbS coexist. Hemoglobin C disease would show HbC predominance rather than HbS, and iron deficiency anemia would not show HbS at all.

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